title: Malignant lymphoma in primary Sjögren's syndrome: an update on the pathogenesis and treatment.
abstract: OBJECTIVE
  Sjögren's syndrome (SS), a chronic autoimmune disorder, particularly compromises the function of exocrine glands. Its association with lymphoma is well documented. Our aim was to systematically review the molecular, clinical, histopathologic, and therapeutic aspects of these SS-related malignant lymphoproliferations.
  
  
  METHODS
  The literature was searched for original articles published between 1968 and 2012 focusing on the risk factors for lymphoma development in Sjögren's syndrome using MEDLINE and PubMed. The search terms we used were "Sjögren's syndrome," "lymphoma," and "risk factors." All papers identified were English-language, full-text papers.
  
  
  RESULTS
  A low-grade marginal-zone lymphoma related to mucosa-associated lymphoid tissue is the commonest lymphoid neoplasia in SS. The majority of SS-associated lymphomas are characterized by localized stage, indolent clinical course, and recurrence in other extranodal sites. Although the transition from a chronic inflammatory condition to malignant lymphoma is a multistep process that is yet poorly understood, there is increasing evidence that chronic antigenic stimulation by an exoantigen or autoantigens plays an essential role in the development of SS-associated lymphoproliferation.
  
  
  CONCLUSIONS
  This review discusses the pathogenetic aspects of lymphomagenesis in SS. Recent advances in the treatment of lymphoma in SS are also stated.
authors.affiliation: Department of Microbiology, School of Medicine, University of Athens, 75M Asias St, 11527 Athens, Greece. Electronic address: jroutsias@med.uoa.gr.
authors.forename: John G
authors.initials: JG
authors.lastname: Routsias
authors.forename: John D
authors.initials: JD
authors.lastname: Goules
authors.forename: Georgios
authors.initials: G
authors.lastname: Charalampakis
authors.forename: Sotiria
authors.initials: S
authors.lastname: Tzima
authors.forename: Aristea
authors.initials: A
authors.lastname: Papageorgiou
authors.forename: Michael
authors.initials: M
authors.lastname: Voulgarelis
mesh_terms: D001402:B-Lymphocytes / Q000473:pathology*; D006801:Humans; D008223:Lymphoma / Q000150:complications / Q000209:etiology / Q000628:therapy*; D012307:Risk Factors; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology
keywords: Autoimmunity; Lymphoproliferation; Non-Hodgkin lymphoma; Salivary glands; Sjögren’s syndrome